Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disorder. It gradually destroys the neurons responsible for muscle movement. Specifically, it affects the upper and lower motor neurons. Over time, ALS leads to almost total paralysis of muscle movement, including breathing. Eventually, the disorder leads to respiratory failure.
Factors that may increase your chance of ALS include:
Symptoms of ALS include:
You will be asked about your symptoms and medical history. A physical exam will be done. There are no tests that can definitively diagnose ALS. Tests may be used to rule out other medical conditions.
Imaging tests may include:
Other tests may include:
Your muscles and nerves may be evaluated. This can be done with electromyogram (EMG)/nerve conduction velocities (NCV).
Your cognitive skills may be assessed. This can be done with neuropsychological testing.
There is currently no cure for ALS.
Treatment may help to reduce or manage symptoms. A combination of treatments may work best. This may include:
Treatment options include:
The drug riluzole has been approved for ALS. The drug may slightly improve functioning, but it does not stop the disease from progressing.
Medications may include:
Supportive care may be needed as ALS progresses, including:
National Institute of Neurological Disorders and Stroke
Amyotrophic Lateral Sclerosis Society of British Columbia
Aggarwal, SP, Zinman L, Simpson E, et al. Clinical trial testing lithium in ALS terminates early for futility. Lancet Neurol. 2010;9(5):481-488.
Amyotrophic lateral sclerosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS. Updated July 25, 2016. Accessed September 30, 2016.
Amyotrophic lateral sclerosis (ALS) fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm. Updated February 1, 2016. Accessed February 12, 2016.
NeuRx Diaphragm Pacing System. Food and Drug Administration website. Available at: http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm. Updated September 6, 2013. Accessed February 12, 2016.
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4/17/2008 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Fornai F, Longone P, Cafaro L, et al. Lithium delays progression of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2008;105(6):2052-2057.
1/14/2011 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Mateen FJ, Carone M, Sorenson EJ. Patients who survive 5 years or more with ALS in Olmsted County, 1925-2004. J Neurol Neurosurg Psychiatry. 2010;81(10):1144-1146.
9/3/2014 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Wippold FJ, Cornelius RS, Broderick DF, et al. American College of Radiology (ACR) Appropriateness Criteria for dementia and movement disorders. Available at: http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/DementiaAndMovementDisorders.pdf. Updated 2014. Accessed February 12, 2016.
Last reviewed February 2016 by Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.